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עמוד בית Mon, 26.08.19

April 2012


Focus
G.M. Weisz, A. Grzybowski and W.R. Albury

The Warsaw Ghetto, in existence from 1940 to 1943, was the largest ghetto in Nazi-occupied Europe. The 400,000–500,000 Jews incarcerated within its walls were deprived not only of food and medicine but also of education. Nonetheless, Jewish physicians served the community as befits their profession, and against all odds obtained permission to conduct a course on sanitary measures to combat epidemics, which they transformed into a veritable, clandestine medical school. This review follows the fate of the school faculty, with an emphasis on the achievements of the survivors.


 
Familial Mediterranean Fever
I. Ben-Zvi, I. Danilesko, G. Yahalom, O. Kukuy, R. Rahamimov, A. Livneh and S. Kivity

Background: Amyloidosis of familial Mediterranean fever (FMF) may lead to end-stage renal failure, culminating in kidney transplantation in some patients.

Objectives: To assess demographic, clinical and genetic risk factors for the development of FMF amyloidosis in a subset of kidney-transplanted patients and to evaluate the impact of transplantation on the FMF course.

Methods: Demographic, clinical and genetic data were abstracted from the files, interviews and examinations of 16 kidney-transplanted FMF amyloidosis patients and compared with the data of 18 FMF patients without amyloidosis.

Results: Age at disease onset and clinical severity of the FMF amyloidosis patients prior to transplantation were similar to FMF patients without amyloidosis. Compliance with colchicine treatment, however, was much lower (50% vs. 98 %). Post-transplantation, FMF amyloidosis patients experienced fewer of the typical serosal attacks than did their counterparts (mean 2214 days since last attack vs. 143 days). Patients with FMF amyloidosis carried only M694V mutations in the FMF gene, while FMF without amyloidosis featured other mutations as well.

Conclusions: Compliance with treatment and genetic makeup but not severity of FMF constitutes major risk factors for the development of amyloidosis in FMF. Transplantation seems to prevent FMF attacks. The protective role of immunosuppressive therapy cannot be excluded.

 

U. Nussinovitch, I. Ben-Zvi and A. Livneh

Background: The association between familial Mediterranean fever (FMF) and increased risk for ventricular arrhythmias is controversial, and data on this subject are meager.

Objectives: To evaluate QT variability index (QTVI) and other repolarization markers associated with arrhythmogenity in patients with amyloidosis of FMF.

Methods: The study group comprised 12 FMF patients with amyloidosis, and 14 age and gender-matched healthy subjects served as the control group. QT measurements were conducted according to accepted procedure, using computerized software for recording and analysis.

Results: No differences were found in clinical and demographic parameters in the study and control groups, except for hypertension which was more common in the FMF amyloidosis group. QTc and power spectral analysis of QT variability parameters were similar in both groups. Nevertheless, QTVI values in FMF amyloidosis patients were significantly higher than in healthy individuals (-1.02 ± 0.38, vs. -1.36 ± 0.32 respectively, P = 0.02).

Conclusions: Compared with healthy controls, amyloidosis of FMF is associated with increased QTVI. It remains unknown whether this finding is solely amyloidosis related and whether it has any prognostic significance.

 

U. Arad, E. Niv, D. Caspi and O. Elkayam

Monogenic periodic fever syndromes are characterized by recurrent episodes of fever, accompanied by localized inflammatory manifestations. Among them, familial Mediterranean fever (FMF) is the most studied and is by far the most prevalent periodic fever syndrome in Israel. We present a diagnostic workup of a patient suffering from a periodic fever syndrome, initially thought to be FMF and characterized by attacks of fever, severe abdominal pain, a migratory erythematous rash and conjunctivitis. The development of periorbital edema presenting as diplopia led to consideration of tumor necrosis factor receptor-1-associated periodic syndrome (TRAPS). Genetic tests confirmed the diagnosis. This case should alert us that even in Israel, a patient with periodic fever, not fully consistent with the typical features of FMF, should be evaluated for other periodic fever syndromes.

Original Articles
A. Achiron, B.-Z. Garty, S. Menascu, D. Magalashvili, M. Dolev, B. Ben-Zeev and O. Pinhas-Hamiel
Background: Multiple sclerosis (MS) occurs in young adults and infrequently appears in childhood.

Objectives: To determine the incidence of MS and describe the clinical, cerebrospinal fluid (CSF) and magnetic resonance imaging (MRI) findings at onset MS in children in Israel.

Methods: Incidence and case-specific data were obtained through the MS Center Database and Israeli Health Statistics Census Data over 15 years, from 1995 to 2009, and compared between patients with childhood (< 12 years), juvenile (> 12 years, < 18 years) and adult (> 18 years) onset MS.

Results: Of 1129 eligible MS patients, we identified 10 (0.89%) with childhood-onset MS, 74 (6.55%) with juvenile-onset MS, and 1045 (92.56%) with adult-onset MS. There were 0 to 3 incident childhood cases/year, leading to an annual incidence of 0.1/100,000 among Israeli children the incidence of juvenile and adult MS was 2.6 and 5.4/100,000, respectively. Neurological presentation among children with MS was optic neuritis, motor weakness or brainstem involvement. CSF oligoclonal immunoglobulin (IgG) were positive in 62.5%. The most frequent MRI finding was the occurrence of ¡Ý 3 periventricular white matter lesions followed by corpus callosum lesions, with 71% co-occurrence. Cervical and thoracic lesions occurred in 33% and 43%, respectively. Time to second neurological event ranged from 0.3 to 4.2 years and none of the patients with childhood MS reached EDSS=6.0 within a mean follow-up period of 8.4 years.

Conclusions: Childhood-onset MS is rare, with an incidence of 0.1/100,000 Israeli children. Childhood MS does not differ significantly from juvenile and adult-onset MS in terms of clinical, laboratory and imaging findings.
R. Nesher, R. Kohen, S. Shulman, B. Siesky, Y. Nahum and A. Harris

Background: Vascular insufficiency is considered to play an important role in the pathogenesis of normal-tension glaucoma (NTG). Autoregulation of blood flow in the eye has been shown to be impaired in NTG, resulting in the inability to compensate for changes in intraocular pressure or blood pressure in order to maintain adequate perfusion. Objectives: To evaluate the occurrence of combined bradycardia-hypotension during 24 hour monitoring of blood pressure and heart rate in patients with NTG.

Methods: Eleven NTG patients participated in the study. All had episodic symptoms of dizziness or lightheadedness, but were confirmed as not having a diagnosis of orthostatic hypotension. Twenty-four hour monitoring was performed with systemic blood pressure and heart rate automatically measured every 20 minutes during daytime and every hour during the night. The cardiac diastolic and systolic double products (dDP and sDP) at each reading were calculated by multiplying the heart rate by the respective blood pressure. dDP < 3600 and sDP < 5400 (corresponding to a heart rate of 60 beats/min and a blood pressure of 60 and 90 mmHg, respectively) were considered abnormally low, and dDP < 2500 and sDP < 4000 (corresponding to a heart rate 50 beats/min and a blood pressure of 50 and 80 mmHg, respectively) were considered severely abnormal.

Results: dDP was abnormally low in all 11 NTG patients on at least one occasion, the majority occurring during the night-time hours, while abnormally low sDP was present in 8 of the 11 patients. The mean cumulative duration of low dDP readings was 4.2 ± 3.2 hours. Severely low dDP readings were observed in six patients.

Conclusions: Abnormally low dDP was recorded in all NTG patients, lasting more than an hour in the majority of cases. Abnormally decreased dDP may represent a state of cardiovascular autonomic dysregulation, resulting in low ocular perfusion in certain NTG patients.

E. Lubart, R. Segal, S. Megid, A. Yarovoy and A. Leibovitz

Background: The QT interval reflects the total duration of ventricular myocardial repolarization. Disturbed QT – either prolonged or shortened – is associated with arrhythmia and is life-threatening.

Objectives: To investigate an elderly population for disturbed QT interval.

Methods: We conducted a cross-sectional study on residents of long-term care wards in a geriatric hospital. Excluded were those with pacemaker, atrial fibrillation or bundle branch block. The standard 12 lead and lead 2 electrocardiograms in the patients’ files were used for the evaluation of QT interval.

Results: We screened the ECGs of 178 residents. QTc prolongation based on the mean 12 ECG leads was detected in 48 (28%), while 45 (25%) had prolonged QTc based on lead L2. Factors associated with QT prolongation were male gender, chronic renal failure and diabetes mellitus. Short QT was found in 7 residents (4%) and was not related to any parameter.

Conclusions: About one-third of the elderly long-term care residents in our study had QT disturbances. Such a considerable number warrants close QT interval follow-up in predisposed patients.

 

Y. Wiener, M. Frank, O. Neeman, Y. Kurzweil, J. Bar and R. Maymon

Background: The triple test serum markers for Down’s syndrome screening may be altered because of various conditions other than chromosomal trisomies.

Objectives: To assess the profile of mid-trimester triple test serum markers in a cohort of women treated with low molecular weight heparin (LMWH) for thrombophilia since the first trimester.

Methods: Women with inherited or acquired thrombophilia treated with LMWH prior to 12 weeks gestation were followed between October 2006 and September 2009 at our obstetric outpatient clinic. The second-trimester screening test for Down syndrome was calculated from the combination of triple serum markers and maternal age, and expressed as a multiple of the gestation specific normal median (MoM). Reference MoM values were calculated from the local population. Data on pregnancy outcome were obtained from patient records.

Results: The median human chorionic gonadotrophin (hCG) level of women with inherited thrombophilia was 0.87 MoM, compared to 0.99 MoM in controls (P = 0.038) and compared to 1.355 MoM in women with acquired thrombophilia (P = 0.034). In contrast, alpha-fetoprotein MoMs did not differ significantly between women with inherited and women with acquired thrombophilia (0.88 vs. 0.99 MoM, P = 0.403).

Conclusions: The triple test serum markers may be altered in thrombophilia patients treated with LMWH. Clinicians should consider offering these patients the first-trimester nuchal translucency test and other sonographic markers that are probably unaffected by the underlying maternal disease and/or treatment modality.

Editorials
הבהרה משפטית: כל נושא המופיע באתר זה נועד להשכלה בלבד ואין לראות בו ייעוץ רפואי או משפטי. אין הר"י אחראית לתוכן המתפרסם באתר זה ולכל נזק שעלול להיגרם. כל הזכויות על המידע באתר שייכות להסתדרות הרפואית בישראל. מדיניות פרטיות
ז'בוטינסקי 35 רמת גן, בניין התאומים 2 קומות 10-11, ת.ד. 3566, מיקוד 5213604. טלפון: 03-6100444, פקס: 03-5753303